AB1309 ANALYSIS OF A MONOCENTRIC COHORT OF IgG4-RELATED DISEASE PATIENTS: GREATER REDUCTION OF THE RESPONDER INDEX IN PHARMACOLOGICALLY-TREATED PATIENTS AS COMPARED TO UNTREATED ONES
Authors: S. Bertocchi, F. Regola, C. Nalli, L. Andreoli, F. Franceschini
Journal: Annals of the Rheumatic Diseases (2022)

Background IgG4-related disease (IgG4-RD) is a systemic disease characterized by lymphoplasmocellular infiltration and storiform fibrosis. Pancreas, biliary tract, lacrimal and salivary glands, lymph nodes and retroperitoneum are frequently involved, although the clinical presentation may vary [1]. Objectives The aim of this study is to retrospectively describe the clinical manifestations of a monocentric cohort of patients with IgG4-RD and analyze their clinical course by applying the validated IgG4-Related Disease Responder Index (RI) [2]. Methods We enrolled 22 IgG4-RD patients classified with 2011 diagnostic criteria [3] and/or 2019 classification criteria [4]. Results 22 IgG4-RD patients (M/F=3.4/1), mostly Caucasian (91%), were diagnosed at a mean age of 59 years (44-74) with a median IgG4 concentration at diagnosis of 239 mg/dl (IQR 106-391 mg/dL). 14/22 (64%) patients fulfilled the 2011 classification criteria, while 8/22 (36%) were classified as possible (2), probable (5) or definitive (1) according to the diagnostic criteria of 2011. Organ involvement at diagnosis (T0) is described in Table 1. The organs most involved were lymph nodes, salivary glands and retroperitoneum. Patients were followed for a median of 49 months (IQR 16-88). Three patients were lost to follow-up. One patient died for pneumonia 96 months after diagnosis. During the follow-up 5 (23%) patients didn’t receive specific treatment, 1 (5%) was treated surgically, 6 (27%) with steroid only, 10 (45%) with steroid and immunosuppressants (with methotrexate and/or rituximab, tocilizumab, mycophenolate, cyclosporine, hydroxychloroquine, azathioprine) and/or surgical resection. A statistically significant reduction in RI between T0 and last follow-up (TLF) was observed in all the patients [from 6 (3-6) to 3 (1-4) p=0.010]. A significant reduction was also observed in the group of pharmacologically treated patients [from 6 (3-6) to 3 (1-4) p=0.035] but not in the group of patients without drug treatment (p = 0.174). Table 1. Organ involvement at diagnosis Organ involvement T0 N. Patients (% ) Pancreas 2 (9) Lymph nodes 14 (64) Salivary glands 13 (59) Lungs 1 (5) Aorta and large blood vessels 4 (18) Retroperitoneum, mediastinum, mesentery 6 (27) Bile duct and liver 3 (14) Kidneys 2 (9) Pachymeninges 1 (5) Orbits and lacrimal glands 3 (14) Paranasal sinuses and / or nasal mucosa 5 (23) Conclusion In our cohort, patients were predominantly male with mean age at diagnosis of 59 years, consistent with literature data [1]. A statistically significant reduction in disease activity defined by reduction of RI was observed in patients who received immunosuppressive therapy. No significant reduction was observed in patients not pharmacologically treated. References [1]Dai Inoue et al. Medicine, 2015 [2]Carruthers M.N. et al. Int J Rheumatol, 2012 [3]Umehara H. et al. Mod Rheumatol, 2012 [4]Wallace Z.S. et al. Ann Rheum Dis, 2020 Disclosure of Interests None declared